pyrimidine synthesis Flashcards

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1
Q

what is the chemical structure of pyrimidines and what are the different types ?

A

contains 1 carbon ring
cytosine
thymidine
uridine

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2
Q

how is UMP made ?

A

start off with glutamine
converted to carbamoyl phosphate
into orotic acid + PRPP
and then into UMP using UMP synthase

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3
Q

where is TMP and CMP derived from ?

A

derived from UMP

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4
Q

what is the enzyme that catalyzes the reaction between glutamine and carbamoyl synthase ?

A

carbamoyl synthetase II
CPS 1

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5
Q

what is the difference between CPS 1 and CCPS 2 ?

A

CPS 1 is found in the liver and is pat of the urea cycle
CPS 2 is involved in pyrimidine synthesis and is found in most cells

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6
Q

what disease is associated with a dysfunction in UMP synthase ?

A

orotic aciduria

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7
Q

what other disease is commonly confused with orotic aciduria ?

A

OTC deficiency

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8
Q

what is the main difference between orotic aciduria and OTC deficiency ?

A

raised ammonia levels in OT defeciency

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9
Q

what is the treatment for orotic aciduria ?

A

uridine

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10
Q

what are the clinical features associated with orotic aciduria ?

A

high levels off orotic acid in urine
megaloblastic anemia

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11
Q

when should i suspect orotic aciduria ?

A

in a baby with megaloblastic anemia that is not responding too folate treatment

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12
Q

what are the sources of the 1 ring formation in pyrimidines ?

A

carbamoyl phosphate
aspartate

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13
Q

what drug is associated with blocking thee conversion of carbamoyl phosphate to orotic acid ?

A

leflunomide

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14
Q
A
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